T HE J OURNAL OF B ONE & J OINT S URGER Y · JBJS . ORG D UPUYTREN D ISEASE : A NATOMY , P ATHOLOGY , V OLUME 89-A · N UMBER 1 · J ANUAR Y 2007 P RESENTATION , AND T REATMENT Dupuytren Disease: Anatomy, Pathology, Presentation, and Treatment By Ghazi M. Rayan, MD An Instructional Course Lecture, American Academy of Orthopaedic Surgeons The disorder called Dupuytren disease volved the palmar fascia, in 1822 Sir There is controversy regarding a rela- has been recognized for approximately Astley Cooper advocated closed fasciot- tionship between Dupuytren disease 400 years. Its presentation, although omy as a treatment for the condition, and seizure disorders 24,25 . The etiology of seemingly rather constant, is actually and in 1831 Guillaume Dupuytren gave Dupuytren disease remains controver- extremely variable, depending on which a detailed anatomic and pathologic de- sial as well, but inflammation, trauma, structures are involved. A thorough scription of the disease and demon- neoplasia, and genetics have been im- plicated as factors 26-31 . The evidence is knowledge of palmar fascial anatomy strated a surgical case in Paris that 4 . By is essential to the understanding of Du- earned him the disease eponym strongest for at least a genetic predispo- puytren disease. There have been re- 1900 there were at least 256 publica- sition. There is a clearly increased inci- cent advances in the pathophysiology tions related to Dupuytren disease and dence in relatives of patients with at least eight books on the subject 2,5-11 . Dupuytren disease, and there has been of Dupuytren disease, and these have added to our knowledge of this disorder Age, gender, geography, and at least one report of identical twins with the disease 13,32 . There seems to be but have not yet changed its treatment. ethnicity influence the disease preva- There are two distinct clinical entities, lence, which has been reported to be as an autosomal dominant transmission classic Dupuytren disease and atypical, low as 2% and as high as 42% 12-14 . Men with variable penetrance. so-called non-Dupuytren palmar fas- are more likely to have it than women 1,2 . These two types differ in cial disease (a ratio of nine to one), and the overall Anatomy presentation, etiology, treatment, and incidence increases with age, with the The radial, ulnar, and central apo- prognosis. Authors of future epidemio- frequency in women catching up to neuroses, palmodigital fascia, and logical and outcome studies should not that in men later in life 15 . The disease digital fascia are all part of the palmar confuse these two clinical entities. Sur- is common in Scandinavia, Great Brit- fascial complex (Fig. 1) 33 . These struc- gical treatment is the conventional and ain, Ireland, Australia, and North tures are involved to varying degrees in most widely used method of managing America. It is uncommon in southern Dupuytren disease. Each structure Dupuytren disease. Europe and South America and rare in can be subdivided. The radial aponeu- The earliest published reference Africa and China 16,17 . rosis has four components: the thenar to the disorder that was later to be Dupuytren disease is associated fascia (an extension of the central apo- called Dupuytren disease was by Felix with diabetes 18-20 . Burge et al. reported a neurosis), the thumb pretendinous Platter, who in 1614 described a case, higher risk of Dupuytren disease in al- band (small or absent), the distal com- attributing the deformity to a flexor coholics, smokers, people with hyperc- missural ligament, and the proximal 3 . In 1777 Henry 34 . The ulnar apo- tendon contracture holesterol, and patients infected with commissural ligament Cline recognized that the disorder in- human immunodeficiency virus 21-23 . neurosis consists of the hypothenar Disclosure: The author did not receive grants or outside funding in support of his research for or preparation of this manuscript. He did not receive payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity. No commercial entity paid or directed, or agreed to pay or direct, any benefits to any research fund, foundation, educational institution, or other charitable or nonprofit organization with which the author is affiliated or associated.
T HE J OURNAL OF B ONE & J OINT S URGER Y · JBJS . ORG D UPUYTREN D ISEASE : A NATOMY , P ATHOLOGY , V OLUME 89-A · N UMBER 1 · J ANUAR Y 2007 P RESENTATION , AND T REATMENT muscle fascia (an extension of the cen- ament of the palmar aponeurosis is ments of two types: four flexor septal tral aponeurosis), the pretendinous proximal and parallel to the natatory canals that contain the flexor tendons band to the small finger (always present ligament and deep to the pretendinous and three web space canals that contain and very substantial), and the abductor bands. Its distal, radial extent is the the common digital nerves and arteries digiti minimi confluence, which is proximal commissural ligament. The and the lumbrical muscles (Fig. 2). enveloped by the fibers of the sagittal transverse ligament of the palmar apo- These septa are inserted in a soft-tissue band 35,36 . The central aponeurosis is the neurosis gives origin to the septa of confluence that consists of five struc- core of Dupuytren disease activity. It is Legueu and Juvara, which protect the tures: the A1 pulley, the palmar plate, a triangular fascial layer with its apex neurovascular structures and provide the sagittal band, the interpalmar plate proximal. Its fibers are oriented longi- an additional proximal pulley to the ligament, and the septa of Legueu and tudinally, transversely, and vertically. flexor tendons. The vertical fibers of the Juvara (Fig. 3). The fascial structures in The longitudinal fibers fan out as pre- central aponeurosis are the minute ver- the palmodigital region are complex. tendinous bands in the three central tical bands of Grapow and the septa of The middle layer of the bifurcated pre- digits, and each bifurcates distally. Legueu and Juvara. The vertical bands tendinous band spirals on its axis nearly 37 . A Each bifurcation has three layers are numerous, small, strong, and scat- 90°, and the peripheral fibers run verti- superficial layer inserts into the dermis, tered along the palmar fascial complex cally adjacent to the metacarpopha- a middle layer continues to the digit as and are most abundant in the central langeal joint capsule 40 . They continue the spiral band, and a deep layer passes aponeurosis 38 . The vertical septa of distally deep to the neurovascular bun- almost vertically and dorsally. The Legueu and Juvara are deep to the pal- dle and natatory ligaments and emerge transverse fibers make up the natatory mar fascia and form fibro-osseous distal to the natatory ligaments to con- 33,39,40 . There are eight ligament located in the distal part of the compartments tinue as the lateral digital sheet. This palm and the transverse ligament of the septa, one radial and one ulnar for each spiral band therefore is the connection palmar aponeurosis. The transverse lig- finger 41 . They form seven compart- between the palmar and digital fascial structures. The proximal fibers of the natatory ligaments run in a transverse plane, but the distal fibers form a “u” and continue longitudinally along both sides of the digit, forming the lateral digital sheet. The lateral digital sheet therefore has deep and superficial con- tributions from the spiral band and the natatory ligament. The neurovascular bundle in the digit is surrounded by four fascial structures: the Grayson liga- ment (palmar), the Cleland ligament (dorsal), the Gosset lateral digital sheet laterally, and the Thomine retrovascular fascia medially and dorsally 42 . Pathophysiology The myofibroblast is the offending cell in Dupuytren disease. It was described 43 and originally by Gabbiani and Majno further studied by Tomasek et al. 44 . The myofibroblast has morphologic charac- teristics of both a fibroblast and a smooth muscle cell, and it can actively contract 45 . The myofibroblast of Du- puytren disease expresses alpha-smooth muscle actin that plays a role in contrac- tion 46 . The Dupuytren myofibroblast Fig. 1 Palmar fascial complex with its five components: the radial aponeurosis (RA), ulnar aponeurosis synthesizes fibronectin, an extracellular (UA), central aponeurosis, palmodigital fascia, and digital fascia. NL = natatory ligament, PA = glycoprotein, which connects myofibro- palmar aponeurosis, and TLPA = transverse ligament of the palmar aponeurosis. (Reprinted, blastic cells together and connects myo- fibroblastic cells to the extracellular with permission, from: Rayan GM. Palmar fascial complex anatomy and pathology in Dupuytren’s stromal matrix with an integrin 47,48 . disease. Hand Clin. 1999;15:75.)
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